Journal
BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY
Volume 35, Issue 2, Pages -Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.beha.2022.101379
Keywords
Myeloproliferative neoplasms (MPN); Accelerated phase MPN; Blast phase MPN; Myelofibrosis
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Myeloproliferative neoplasms (MPN) carry the risk of transformation into blast phase (MPN-BP) or accelerated phase (MPN-AP). Primary myelofibrosis (PMF) has the highest risk of blastic transformation, followed by polycythemia vera (PV) and essential thrombocythemia (ET). A three-tiered model can be used to determine the risk of leukaemic transformation (LT) in PMF. Current treatment options include chemotherapy, hypomethylating agents, and stem cell transplantation.
Myeloproliferative neoplasms (MPN) have an inherent risk of transformation into blast phase (MPN-BP) or accelerated phase (MPN-AP) which is characterized by presence of >= 20% or 10-19% peripheral blood or bone marrow blasts, respectively. Primary myelofibrosis (PMF) is associated with the highest risk of blastic transformation (14.2%), followed by polycythemia vera (PV) (6.8%) and essential thrombocythemia (ET) (3.8%). Risk of leukaemic transformation (LT) in PMF can be determined by a three-tiered model based on presence of IDH1 mutation, circulating blasts >= 3%, SRSF2 mutation, age >70 years, ASXL1 mutation, and moderate/severe anemia with high, intermediate, and low risk groups (LT incidence 57%, 17%, and 8%, respectively). Currently, treatment of MPN-AP/BP includes acute myeloid leukaemia (AML)-like induction chemotherapy or hypomethylating agents alone or in combination with venetoclax and/or ruxolitinib. In transplant-eligible patients, our goal is to achieve complete remission with or without count recovery, before proceeding with allogeneic stem cell transplantation, which is the only modality associated with long-term survival. In the current review, we discuss our diagnostic, prognostic, and therapeutic approach to patients with MPN-AP/BP.
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